Extensive thrombosis following central venous catheterization in a neonate with homozygous protein C deficiency presenting purpura fulminans
Mozhgan Shahian, Omidreza Zekavat, Mehran Karimi
Case Rep Clin Pract Rev 2006; 7:107-110
Background: Homozygous protein C deficiency is a rare inherited disorder associated with neonatal purpura
fulminans, recurrent venous thrombosis and increasing venous thrombosis in high-risk situations such as central venous catheterization.
Case Report: The presented case is a homozygous protein C deficient neonate with purpura fulminans on
her buttocks and the lower part of the abdomen from 24 hours after birth. The patient had a protein C level of less than 1% and her parents were cases of asymptomatic heterozygous protein C deficiency. Since purified protein C concentrate was not available, administration of fresh frozen plasma was commenced for the patient leading to a relative improvement in necrotic skin lesions. Because of multiple venous punctures and poor peripheral venous access, jugular
vein catheterization was done to establish an efficient intravenous line for administration of
fresh frozen plasma. Finally, after a few days, superior vena cava syndrome and disseminated
intravascular coagulation developed, and the patient expired. Autopsy showed extensive thrombosis of central veins.
Conclusions: When protein C concentrate is not available, newborns with homozygous protein C deficiency are at a greater risk of central vein line-related deep venous thrombosis, which may occur as
a short-term complication; therefore, in these patients, central vein catheterization should be
avoided as far as possible, and be considered only as the final therapeutic decision for poor
Keywords: neonate, Protein C Deficiency, purpura fulminans, central venous catheterization